Materiały źródłowe

• #1 Guidelines for the diagnosis and management of carcinoid tumours. Part 1: The gastrointestinal tract. A statement from a Canadian National Carcinoid Expert Group

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1891174/

  Carcinoid tumours are relatively rare and, in general, slow growing. […] Proper patient management can lead to cure, particularly if the tumour can be fully resected, or to long-term palliation with medical treatment or cytoreductive surgery, or both, with significant prolongation of survival. […] A good understanding of the use of somatostatin analogues to achieve effective symptomatic control and of the importance of adequate follow-up and cardiac monitoring to prevent or effectively treat cardiac complications can contribute significantly to optimal control of this complex disease, ultimately improving the quality of life of affected patients. […] Debulking (cytoreductive surgery) is a mainstay of therapy. Resection of the tumour can be curative. […] Somatostatin analogues such as octreotide and lanreotide have hormone-blocking properties and can facilitate the surgical procedure, provide symptom control, and reduce the risk of carcinoid crises and other severe events. They are first-line agents in the management of carcinoid syndrome.

• #1 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044

  Treatment for a carcinoid tumor depends on the tumor’s location, whether cancer has spread to other areas of the body, the types of hormones the tumor secretes, your overall health and your own preferences. […] Carcinoid tumor treatment options may include: […] Surgery. When detected early, a carcinoid tumor may be removed completely using surgery. If carcinoid tumors are advanced when discovered, complete removal may not be possible. In some situations, surgeons may try to remove as much of the tumor as possible, to help control signs and symptoms. […] Medications to control excess hormones. Using medications to block hormones secreted by the tumor may reduce the signs and symptoms of carcinoid syndrome and slow tumor growth. Octreotide (Sandostatin, Bynfezia Pen) and lanreotide (Somatuline Depot) are given as injections under the skin. Side effects from either medication may include abdominal pain, bloating and diarrhea. Telotristat (Xermelo) is a pill that is sometimes used in combination with octreotide or lanreotide to further try to improve the symptoms of carcinoid syndrome.

• #1 Carcinoid Syndrome Treatment – NETRF

  https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/carcinoid-syndrome-treatment/

  There are a variety of carcinoid syndrome treatments, including somatostatin analogs and other medications, liver-directed therapies, surgery, and chemotherapy. […] Somatostatin analogs include Octreotide and Lanreotide. […] Both Octreotide and Lanreotide have similar effectiveness and provide symptom relief in 50% to 70% of people with carcinoid syndrome. […] Surgical treatments aim to remove or reduce the volume of NETs, causing carcinoid syndrome. Surgery is generally reserved for patients in which 90% of the tumor can be removed and who do not have compromised liver function or extensive liver metastases. […] If liver metastases can be surgically removed, it often leads to the improvement of carcinoid syndrome symptoms. […] For patients with heavy tumor burden and metastases, tumor debulking (removing large portions of the tumor) may improve symptoms as well as extend and improve the overall health of the patient.

• #1 Carcinoid Tumor Treatment & Management: Medical Care, Surgical Care

  https://emedicine.medscape.com/article/986050-treatment

  In localized tumors, surgical resection can result in cure, with 70% to more than 90% survival rate. […] When total resection is not possible, debulking may provide symptomatic relief. […] In selected cases, cryotherapy can be effective. Bronchoscopic cryotherapy has been successfully applied in treatment of isolated endoluminal carcinoid tumor in an adult patient. […] For hepatic tumors, surgical ligation of the hepatic artery can potentially deprive blood supply to the tumor cells and cause necrosis while preserving most of the normal live cells. However, over time new blood vessels develop and restore circulation. […] In patients with tumors less than 1 cm located in the appendix, appendectomy is the treatment of choice. More extensive surgery is indicated for tumors larger than 2 cm, lymphatic invasion, lymph node involvement, mesoappendix infiltration, positive resection margins, and cellular pleomorphism with a high mitotic index.

• #1 Carcinoid Tumors: Causes, Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/cancer/carcinoid-tumors

  Once your doctor knows what kind of carcinoid tumor you have and where in your body it is, you can start to make an action plan. […] You may have surgery to remove all or part of the tumor. The type you get depends on where your cancer is located. […] The surgeon will make a cut in the skin and remove the tumor, along with some of the tissue around it. If the tumor is in the rectum, they may try a method that uses an electric current to heat and destroy it. This is called fulguration. […] Your surgeon may remove the tumor and parts of the airway above and below it. This is called a sleeve resection. The airway is reconnected after the surgery. For a larger tumor, the surgeon may remove a piece of your lung or all of it. They may also take out some lymph nodes to stop the tumor from spreading.

• #1 Lung Carcinoid Tumor: Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/14783-carcinoid-tumors-of-the-lungs

  Surgery is the most common treatment. […] Your treatment plan will depend on the size of the tumor, its location and your overall health. Many lung carcinoid tumors can be treated with surgery alone. If cancer has spread to other organs or if the entire tumor cant be removed, you may need additional treatments. […] Your treatment options may include: Surgery. A surgeon may remove part of your lung with a lobectomy or wedge resection. In rare cases, they might remove your entire lung (pneumonectomy). Sometimes, surgeons also remove tumors that have spread to other organs in your body. […] Radiation therapy. Providers usually use external-beam radiation to treat carcinoid tumors. They also sometimes use brachytherapy (internal radiation) where they place pellets or rods of radiation close to the tumor.

• #1 Carcinoid syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/carcinoid-syndrome/diagnosis-treatment/drc-20370672

  Treating carcinoid syndrome involves treating your cancer and may also involve using medications to control your specific signs and symptoms. […] Treatments may include: […] Surgery to remove your cancer or most of your cancer may be an option. […] Injections of the medications octreotide (Sandostatin) and lanreotide (Somatuline Depot) may reduce the signs and symptoms of carcinoid syndrome, including skin flushing and diarrhea. A drug called telotristat (Xermelo) can be combined with these drugs to control diarrhea caused by carcinoid syndrome. […] Peptide receptor radionuclide therapy (PRRT) combines a drug that seeks out cancer cells with a radioactive substance that kills them. In PRRT for carcinoid tumors, the drug is injected into your body, where it travels to the cancer cells, binds to the cells and delivers the radiation directly to them. This therapy is used in people who have advanced cancer that hasn’t responded to other treatments.

• #1 Management of carcinoid syndrome: a systematic review and meta-analysis in: Endocrine-Related Cancer Volume 26 Issue 3 (2019)

  https://erc.bioscientifica.com/view/journals/erc/26/3/ERC-18-0495.xml

  Carcinoid syndrome (CS) is a debilitating disease caused by functional neuroendocrine tumors. Several treatment options are available to alleviate the hormonal symptoms, but their relative efficacy is unknown. […] The somatostatin analogs octreotide and lanreotide induced symptomatic improvement in 65-72% and biochemical response in 45-46% of patients. An increase in dose or frequency or interclass switch led to a reduction of flushes and/or diarrhea in 72-84% of cases. […] Retrospective, institutional series showed that liver-directed therapy can improve symptoms in 82% of CS patients with a liver-dominant disease. The serotonin synthesis inhibitor telotristat ethyl reduced bowel movements in 40% of patients with diarrhea refractory to somatostatin analogs. Interferon-alpha controlled CS symptoms in 45-63% of cases.

• #1 Carcinoid Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK448096/

  Telotristat is an FDA-approved agent to treat refractory diarrhea not responding to SSA. It inhibits the enzyme tryptophan hydroxylase, which catalyzes a rate-limiting step in the conversion of the tryptophan to serotonin. […] PRRT is a targeted systemic treatment for advanced neuroendocrine tumors that express somatostatin receptors, utilizing radiolabeled somatostatin analogs (eg, lutetium-177) to deliver localized ionizing radiation to tumor cells. […] Surgery is critical in managing patients with disease that is amenable to cytoreduction. In metastatic midgut tumors, removing over 70% of the tumor burden improves symptoms and overall survival, while resection can be curative in other tumor sites (eg, the bronchus). […] For patients with inoperable hepatic metastases, liver-directed therapies can reduce morbidity and improve quality of life. Options include percutaneous hepatic transarterial embolization, radioembolization with yttrium-90 microspheres, and ablation.

• #1 Emerging Treatment Options for Carcinoid Syndrome – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/september-2016/emerging-treatment-options-for-carcinoid-syndrome/

  Carcinoid tumors fall into the broader category of neuroendocrine tumors. […] The standard of care has been treatment with somatostatin analogues, which were initially developed in the 1980s. They have had a very beneficial impact on patients with carcinoid syndrome. They decrease levels of serotonin and improve quality of life, and more recently have also been shown to slow tumor growth. […] A recent development has been the introduction of telotristat etiprate, which is a tryptophan hydroxylase inhibitor. […] In clinical trials, the side effect profile of telotristat etiprate was very favorable. Telotristat etiprate does not cross the blood-brain barrier. It is therefore an exciting development. […] Future research should focus on how to best use telotristat etiprate to improve quality of life. In addition, it should also determine whether the use of this therapy can ameliorate some of the longer-term consequences of high serotonin levels.

• #1 Intestinal Carcinoid Tumor Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/276837-treatment

  A randomized, placebo-controlled phase 3 trial found that everolimus plus octreotide long-acting repeatable (LAR) improved progression-free survival in patients with advanced neuroendocrine tumors associated with intestinal carcinoid syndrome. […] The US Food and Drug Administration (FDA) has approved the radiolabeled somatostatin analog Lutathera (lutetium Lu 177 dotatate) for the treatment of adult patients with somatostatin receptor positive gastroenteropancreatic neuroendocrine tumors (GEP-NETs), which would include intestinal carcinoids. […] Telostristat ethyl (Xermelo) is approved by the FDA for carcinoid syndrome diarrhea in combination with SSA therapy in adults inadequately controlled by an SSA. […] Patients with problematic diarrhea usually benefit from antidiarrheal medication. Cyproheptadine and histamine-2 receptor blockers may work because they suppress the production of vasoactive amines or block their peripheral effects.

• #1 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044

  Treatment for cancer that spreads to the liver. Carcinoid tumors commonly spread to the liver. Treatments may include surgery to remove part of the liver, blocking blood flow to the liver (hepatic artery embolization), and using heat and cold to kill cancer cells. Radiofrequency ablation delivers heat treatments that cause carcinoid tumor cells in the liver to die. Cryoablation uses cycles of freezing and thawing to kill cancer cells.

• #1 Carcinoid syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/carcinoid-syndrome/diagnosis-treatment/drc-20370672

  In a procedure called hepatic artery embolization, a doctor inserts a catheter through a needle near your groin and threads it up to the main artery that carries blood to your liver (hepatic artery). The doctor injects particles designed to clog the hepatic artery, cutting off the blood supply to cancer cells that have spread to the liver. […] Radiofrequency ablation delivers heat through a needle to the cancer cells in the liver, causing the cells to die. Cryotherapy is similar, but it works by freezing the tumor. […] Chemotherapy uses strong drugs to kill cancer cells. Chemotherapy drugs can be given through a vein (intravenously) or in pill form, or both methods can be used.

• #1 Carcinoid Tumors: Causes, Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/cancer/carcinoid-tumors

  It uses drugs to stop cancer cells from growing. You might take these in the form of pills or get them put into you through a vein. Your doctor may use this treatment if your disease has spread. Side effects of chemo include nausea, vomiting, hair loss, loss of appetite, and a higher risk for infections. […] It’s a treatment used to treat a carcinoid tumor that has spread to the liver. Chemo drugs are delivered straight to the liver through a tube called a catheter that a doctor inserts into an artery. The drug stops blood flow to the tumor. […] It stops the tumor from making extra hormones. The drugs octreotide and lanreotide treat GI carcinoid tumors. You get them through a shot. […] It helps your body’s immune system fight the cancer better. You may get a drug such as alpha-interferon.

• #1 Carcinoid Tumor Treatment & Management: Medical Care, Surgical Care

  https://emedicine.medscape.com/article/986050-treatment

  If metastasis of carcinoid tumor has occurred and in cases where surgical excision is not suitable, consider treatment with currently recommended chemotherapy. […] Chemotherapeutic agents currently used in clinical trials to palliate metastatic carcinoid disease include the following: Alkylating agents, Doxorubicin, 5-Fluorouracil, Dacarbazine, Actinomycin D, Cisplatin, Etoposide, Streptozotocin, Interferon alfa, Somatostatin analogs with a radioactive load, Experimental agents such as 177Lu-Dotatate. […] A combination of the agents listed above is typically used. […] Chemoembolization of hepatic artery for treatment of metastatic carcinoid tumor has been widely used in adults. […] In one study, 8 adults with carcinoid tumor metastatic to liver were treated with intra-arterial 5-fluorouracil and embolization of hepatic tumors with bovine collagen fiber admixed with iohexol, cisplatin, mitomycin C, and doxorubicin. This treatment resulted in symptomatic relief and tumor regression in 4 patients and stabilized the disease in the rest of the patients.

• #1 Carcinoid syndrome: update on the pathophysiology and treatment | Clinics

  https://www.elsevier.es/en-revista-clinics-22-articulo-carcinoid-syndrome-update-on-pathophysiology-S1807593222011310

  The main pillar of treatment for CS is the use of somatostatin analogues, such as octreotide and lanreotide. Approximately 80% of well-differentiated tumors express somatostatin receptors in the NET cell surface. Octreotide and lanreotide bind to somatostatin receptors and inhibit the secretion of several hormones and vasoactive substances, thus improving flushing and diarrhea symptoms in over 80% patients with CS. […] Unfortunately, all patients experience symptomatic progression of CS after a median of several months to years. In this scenario, several therapeutic options have been successfully tested and are discussed below. […] Telotristat ethyl was recently approved by the US Food and Drug Administration. Together with octreotide, telotristat reduces the frequency of diarrhea. […] An alternative to somatostatin analogues for the treatment of refractory diarrhea and flushing is interferon alpha, which, in retrospective studies, has been shown to provide symptom relief in up to 40-50% patients.

• #1 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044

  Chemotherapy. Chemotherapy uses strong drugs to kill tumor cells. It can be given through a vein in your arm or taken as a pill. Chemotherapy is sometimes recommended for treating advanced carcinoid tumors that can’t be removed with surgery. […] Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within tumor cells. By blocking these abnormalities, targeted drug treatments can cause tumor cells to die. Targeted drug therapy is usually combined with chemotherapy for advanced carcinoid tumors. […] Drugs that deliver radiation directly to the cancer cells. Peptide receptor radionuclide therapy (PRRT) combines a drug that seeks out cancer cells with a radioactive substance that kills them. In PRRT for carcinoid tumors, the drug is injected into your body, where it travels to the cancer cells, binds to the cells and delivers the radiation directly to them. This therapy may be an option for people with advanced carcinoid tumors.

• #1 What are neuroendocrine tumors, and how are they treated? | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/neuroendocrine-tumors–9-things-to-know.h00-159379578.html

  One Phase III trial (NETTER-2) recently showed it could reduce the risk of disease progression by up to 72% when used as a front-line therapy for patients with more aggressive tumors. […] We believe that patients benefit most when they get the right treatment at the right time with doctors who specialize in treating these complex diseases.

• #1 Chemotherapy and Other Drugs for Gastrointestinal Carcinoid Tumors | American Cancer Society

  https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/treating/chemotherapy.html

  Chemotherapy (chemo) uses anti-cancer drugs that are injected into a vein or a muscle or taken by mouth to kill cancer cells. These drugs enter the blood and reach almost all areas of the body, making this treatment useful for some types of cancers that have spread. […] Unfortunately, gastrointestinal (GI) carcinoid tumors often do not respond well to chemo. Because of this, chemo generally is used only for tumors that have spread to other organs, are causing severe symptoms, have not responded to other medicines or are high grade (grade 3). […] Some tumors, especially high-grade tumors, may be treated with more than one drug. For these, combinations of 5-FU plus streptozocin, 5-FU plus doxorubicin or oxaliplatin plus capecitabine may be used. […] For people with metastatic GI carcinoid tumors, several medicines can help control symptoms and tumor growth.

• #1 Chemotherapy and Other Drugs for Gastrointestinal Carcinoid Tumors | American Cancer Society

  https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/treating/chemotherapy.html

  These drugs are related to somatostatin, a natural hormone that seems to help slow the growth of neuroendocrine cells. They are especially useful in people who have carcinoid syndrome (facial flushing, diarrhea, wheezing, rapid heart rate) and in people whose tumors show up on a somatostatin receptor scintigraphy (SRS) scan or gallium-68 Dotatate scans. […] This drug is helpful in treating the symptoms of carcinoid syndrome. Sometimes octreotide can temporarily shrink carcinoid tumors, but it does not cure them. […] The targeted drug, everolimus (Afinitor), has been shown to help treat advanced GI carcinoid tumors. It can be used with or without somatostatin drugs, such as octreotide. […] Interferon-alfa is sometimes helpful in shrinking or slowing the growth of metastatic GI carcinoid tumors and improving symptoms of carcinoid syndrome.

• #1 Carcinoid Tumor Treatment & Management: Medical Care, Surgical Care

  https://emedicine.medscape.com/article/986050-treatment

  Octreotide, a somatostatin analog, is highly effective in reducing symptoms; however, in the pediatric age group, stunted linear growth is of concern. Survival advantage with the use of this drug has not yet been proven. […] A randomized, double-blind, placebo-controlled, phase 3 study showed that using everolimus in conjunction with octreotide improved progression-free survival in patients with low-grade and intermediate-grade advanced neuroendocrine tumors. […] In situ targeted therapy with somatostatin analogs (eg, octreotide attached to a radioactive load using yttrium-90 or111 indium-labeling agents) provides promise for patients with unresectable tumors. This therapy is currently used on an experimental basis in adults and children. […] If feasible, the treatment of choice is surgical excision which is associated with an excellent prognosis, especially in appendiceal tumors.

• #1 Research Breakthroughs for Neuroendocrine and Carcinoid Tumors | Dana-Farber Cancer Institute

  https://www.dana-farber.org/cancer-care/treatment/gastrointestinal/programs/neuroendocrine-carcinoid/research

  Dana-Farber investigators are leading an array of studies to uncover new treatments for patients with carcinoid tumors. […] The study results revealed that everolimus is effective when used to target this set of tumors, and led its approval by the Food and Drug Administration in 2016 as a treatment for carcinoid tumors. […] An international study evaluated the drug telotristat in treating carcinoid syndrome, a set of severe symptoms that can occur in patients with carcinoid tumors. […] After publishing findings in July 2014 in Endocrine-Related Cancer revealing that this drug was safe and impactful, the team helped to lead a pivotal, international clinical trial to test this drug in patients with carcinoid syndrome who had not responded to standard treatments, and found that telotristat helped relieve some of the most challenging symptoms.

• #1 Research Breakthroughs for Neuroendocrine and Carcinoid Tumors | Dana-Farber Cancer Institute

  https://www.dana-farber.org/cancer-care/treatment/gastrointestinal/programs/neuroendocrine-carcinoid/research

  Initial studies of the angiogenesis inhibitor sunitinib in pancreatic neuroendocrine tumors led to its approval as a standard treatment for these tumors in 2011. […] A multi-institution cooperative clinical trial tested everolimus in combination with another angiogenesis inhibitor called bevacizumab. […] They discovered that this combination was effective in patients with advanced or metastatic pancreatic neuroendocrine tumors, and that together, the two drugs helped to improve progression-free survival and response rates. […] The team’s studies using this model also revealed that the carcinoid tumor microenvironment the cellular environment around a tumor appears to play a significant role in driving carcinoid tumor development and growth. […] This finding changed the way that Dana-Farber investigators study this disease, expanding their focus from mutations within cancer cells to the surrounding tissue.

• #1 Carcinoid Tumors: Causes, Symptoms, Diagnosis, and Treatment

  https://www.webmd.com/cancer/carcinoid-tumors

  If your cancer has spread there, your surgeon may remove the areas where the tumors are. This is called liver resection. […] Before your operation, make sure your surgeon knows if you have carcinoid syndrome, because your tumor can release a dangerous amount of hormones during surgery. You’ll get medicine beforehand to stop this from happening. […] Your doctor might also try some other treatments along with surgery to make it work better. Or they might suggest them if you can’t have surgery. Some of these choices are: […] It uses high-energy X-rays to kill cancer cells. Most of the time you get this from a machine outside your body. Or the doctor can implant radioactive seeds inside your body, near the tumor. Side effects can include fatigue and redness in the treated area. If you get radiation to the neck or throat, you may have a sore throat, cough, shortness of breath.

• #1 Carcinoid Tumours: Causes, Symptoms and Treatment | Doctor

  https://patient.info/doctor/carcinoid-tumours

  Treatment of carcinoid tumours […] Treatment is usually based on the size of the tumour. Surgical resection (local resection with node clearance) when possible is the treatment of choice. Surgery should be considered for patients with liver metastases and potentially resectable disease. Options for nonresectable disease include somatostatin analogues – eg, octreotide (which blocks 5-HT release), biotherapy, targeted radionuclide therapy, radiofrequency ablation therapy and chemotherapy. For advanced metastatic disease, somatostatin analogue therapy and surgical debulking provide the best symptomatic relief and may improve survival. Chemotherapy drugs currently being assessed in trials to palliate metastatic carcinoid disease include alkylating agents, doxorubicin and 5-fluorouracil. Interferon alfa is a useful additive therapy when symptoms of carcinoid syndrome do not resolve with a somatostatin analogue alone. External beam radiotherapy may relieve bone pain from metastases. Chemoembolisation of the hepatic artery may provide effective, short-term relief of symptoms due to hepatic metastasis. Liver transplantation can be considered in selected cases – eg, young patients without documented spread outside the liver and resected primary tumour. For patients with carcinoid valvular heart disease, surgical intervention can lead to improved prognosis and reduce the symptoms of heart failure.

• #1 Carcinoid Syndrome due to Neuroendocrine Tumors – Hormonal and Metabolic Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/hormonal-and-metabolic-disorders/gastrointestinal-and-pancreatic-neuroendocrine-tumors/carcinoid-syndrome-due-to-neuroendocrine-tumors

  Sometimes tumors are removed surgically. […] People may need to take medications to control symptoms. […] The medications octreotide and lanreotide can relieve flushing symptoms. Some forms of these medications can be given only once a month. Other treatments for flushing include phenothiazines (such as prochlorperazine) and histamine-blocking drugs such as famotidine. Rarely, phentolamine is used to control flushing in people with carcinoid syndrome. Prednisone is sometimes given to people with neuroendocrine tumors of the lungs who have episodes of severe flushing. […] Diarrhea may be controlled with loperamide, codeine, tincture of opium, diphenoxylate, or cyproheptadine. […] Pellagra may be prevented by ensuring adequate protein in the diet and by taking niacin. […] When a neuroendocrine tumor is restricted to a specific area, such as the appendix, small intestine, rectum, or lungs, surgical removal may cure the disease. If the tumor has spread to the liver, surgery rarely cures the disease but may help relieve symptoms. Other treatments, such as embolization, in which substances are injected into the tumor through blood vessels in the liver, also can help liver tumors. Neuroendocrine tumors grow so slowly that even people whose tumors have spread often survive for 10 to 15 years. […] Several medications such as everolimus also may be helpful. Chemotherapy is usually not helpful, but certain regimens (streptozocin with fluorouracil and cyclophosphamide) are often tried in people whose disease has spread. Radiation therapy is not useful.

• #1 Treatment Options – Carcinoid Cancer Foundation

  https://www.carcinoid.org/for-patients/treatment/treatment-options/

  It has been shown that giving niacin supplementation to neuroendocrine cancer patients not only resolves several common symptoms of carcinoid/neuroendocrine tumors(NETs) and pellagra, such as skin lesions and diarrhea/ steatorrhea, but also generally improves the health of the NET patients. Therefore, our results warrant that niacin status should be determined for all NET patients, so that active niacin replacement could be provided to biochemically niacin-deficient patients. In areas of the world where preformed niacin is not added to the food supply and screening of niacin status is not possible, all carcinoid patients should be supplemented with niacin as a preventative therapy. […] For comprehensive information regarding the use of the somatostatin analogue Sandostatin in the forms of subcutaneous injection (SC), long-acting injection(Sandostatin LAR) and continuous subcutaneous infusion via a pump (pump-based therapy) read the following newly published paper by Dr. Eugene Woltering (2005) A discussion on the utility of various routes of administration of octreotide acetate.

• #1 Carcinoid Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK448096/

  Carcinoid crisis is a potentially life-threatening complication characterized by severe hemodynamic instability, flushing, bronchoconstriction, and hypotension, often triggered by surgery, anesthesia, or tumor necrosis. It typically occurs in patients with high levels of circulating vasoactive peptides, eg, serotonin. Prophylactic administration of octreotide is recommended in patients not on SSA as part of treatment.

• #1 Treatment Options – Carcinoid Cancer Foundation

  https://www.carcinoid.org/for-patients/treatment/treatment-options/

  Octreotide has largely replaced the use of other drugs both for symptomatic control and acute treatment of the symptoms associated with carcinoid syndrome. […] We suggest that hypertensive as well as hypotensive carcinoid crises respond to octreotide and that this agent should be considered for prophylactic and emergency use in all carcinoid syndrome patients prior to and during anesthesia and surgery. […] Hepatic Artery Embolization is safe, provides good control of hormonal symptoms, and prolongs survival in biochemically responsive patients. It is a valuable palliative option for patients with midgut carcinoid syndrome due to liver metastases and can be repeated in patients with a favourable response to the first procedure. […] High-dose 111In-Pentetreotide (~500 mCi/patient) is now offered in the U.S. for therapy in somatostatin receptor expressing neuroendocrine tumors. This therapy can be applied to the category of neuroendocrine tumors which include Carcinoid, Islet Cell Carcinoma of the Pancreas, Oat Cell Carcinoma of the Lung, and Medullary Thyroid Carcinoma.

• #1 Treating Gastrointestinal Carcinoid Tumors | American Cancer Society

  https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/treating.html

  If you’ve been diagnosed with a gastrointestinal (GI) carcinoid tumor, your cancer care team will discuss your treatment options with you. It’s important to weigh the benefits of each treatment option against the possible risks and side effects. […] The main types of treatment for GI carcinoid tumors are: Surgery for Gastrointestinal Carcinoid Tumors, Chemotherapy and Other Drugs for Gastrointestinal Carcinoid Tumors, Radiation Therapy for Gastrointestinal Carcinoid Tumors. […] In some cases, doctors may recommend combining two or more types of treatment. […] Its important to discuss and understand all your treatment options, including their goals and possible side effects, with your doctors to help make the decision that best fits your needs. […] Clinical trials are carefully controlled research studies that are done to get a closer look at promising new treatments or procedures. Clinical trials are one way to get state-of-the-art cancer treatment.

• #1 Carcinoid Syndrome, Symptoms, Diagnosis, Treatment

  https://crinetics.com/carcinoid-syndrome-symptoms-diagnosis-treatment/

  Depending on the patient’s response, a combination of therapies may be used. A number of different side effects are possible. Please refer to product labeling for a complete list of side effects associated with any of these therapies. […] Crinetics is developing paltusotine for the treatment of NETs and carcinoid syndrome. Paltusotine establishes a new class of oral, selective, nonpeptide, somatostatin receptor type 2 (SST2) agonists. This once-daily oral investigational treatment is the first agent in its class with reported clinical trial results for the treatment of acromegaly.

• #1 Treating Gastrointestinal Carcinoid Tumors | American Cancer Society

  https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/treating.html

  You may hear about alternative or complementary methods to relieve symptoms or treat your cancer that your doctors havent mentioned. […] Be sure to talk to your cancer care team about any method you are thinking about using. […] Whether you are thinking about treatment, getting treatment, or not being treated at all, you can still get supportive care to help with pain or other symptoms. […] For some people, when treatments have been tried and are no longer controlling the cancer, it could be time to weigh the benefits and risks of continuing to try new treatments. […] Remember that even if you choose not to treat the cancer, you can still get supportive care to help with pain or other symptoms.

• #1

  https://winshipcancer.emory.edu/cancer-types-and-treatments/neuroendocrine-tumors/treatment.php

  Neuroendocrine tumors are complex and require a highly skilled team of medical oncologists, radiation oncologists, surgeons, nuclear medicine specialists and others working together to effectively treat the disease. […] In many cases, it also requires specialized neuroendocrine tumor treatments that arent offered everywhere. […] At Winship Cancer Institute of Emory University, we have the technology and expertise to treat all types of neuroendocrine cancers. […] The gastrointestinal cancer experts at Winship use the latest technology and advanced testing to analyze your neuroendocrine cancer at every level. […] Once your personalized neuroendocrine cancer treatment plan is created, your team will discuss it with you, and treatment at Winship will be scheduled right away. […] We can offer you access to the latest neuroendocrine tumor treatments you wont find at other cancer centers.

• #1 About Neuroendocrine and Carcinoid Tumors Program | Dana-Farber Cancer Institute

  https://www.dana-farber.org/cancer-care/treatment/gastrointestinal/programs/neuroendocrine-carcinoid

  At the Neuroendocrine and Carcinoid Tumors Program at Dana-Farber Brigham Cancer Center, our goal is to improve the lives of people with neuroendocrine tumors by offering patients a comprehensive range of services based on specialized treatments and innovative research. Our novel therapies and clinical trials give our patients access to the latest and most sophisticated therapies tailored to their tumor. […] In the Neuroendocrine and Carcinoid Tumors Program, our experts specialize in the treatment of neuroendocrine tumors, while continually developing new and better therapies for the future. […] Our specialists work closely together to offer coordinated, advanced treatments, including access to novel therapies that were discovered in our own labs, and several clinical trials. […] We offer an approach to treatment that includes: A team of oncologists specializing in neuroendocrine tumors. A multidisciplinary approach to care based on each patient’s unique situation. Highly advanced diagnostic procedures. Complex surgical procedures. Interventional radiology techniques, including embolization. Access to leading-edge clinical trials. Pathologic and molecular testing of tumor specimens. Genetic testing and counseling for families with a cancer history. Easy access and timely appointments.

• #1 Treatments for neuroendocrine tumours (NETs) | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/treatment

  If neuroendocrine tumours (NETs) spread, they often spread to the liver (called liver metastases). Liver directed therapy directly targets the cancer in the liver and is often used to treat NETs that have spread to the liver. Liver directed therapy is mainly used when surgery can’t be done. […] Neuroendocrine cancer behaves differently in each person, and a standard follow-up schedule would not work for everyone. People with neuroendocrine cancer should talk to their doctor about a follow-up plan that suits their individual situation. Follow-up care is often shared among the cancer specialists, such as the surgeon, oncologist and endocrinologist, and your family doctor.

• #2 Guidelines for the diagnosis and management of carcinoid tumours. Part 1: The gastrointestinal tract. A statement from a Canadian National Carcinoid Expert Group

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1891174/

  Cytoreductive surgery, which aims to control symptoms and improve survival by removing or destroying the tumour, is a mainstay in the management of carcinoid tumour. […] Surgery is the only way to obtain a complete cure. […] Octreotide administration is crucial before an invasive procedure. […] For liver metastases, surgical intervention can be palliative. […] Embolization, either alone or in combination with intra-arterial chemotherapy (chemoembolization), can reduce clinical symptoms and liver metastases. […] Systemic treatment for carcinoid tumours includes therapy with somatostatin analogues, interferon alfa, and cytotoxic agents. […] Somatostatin analogues can relieve symptoms and reduce hormone levels. […] Interferon alfa inhibits protein and hormone synthesis in tumour cells, inhibits angiogenesis, and stimulates the immune system.

• #2 Chemotherapy and Other Drugs for Gastrointestinal Carcinoid Tumors | American Cancer Society

  https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/treating/chemotherapy.html

  These drugs are related to somatostatin, a natural hormone that seems to help slow the growth of neuroendocrine cells. They are especially useful in people who have carcinoid syndrome (facial flushing, diarrhea, wheezing, rapid heart rate) and in people whose tumors show up on a somatostatin receptor scintigraphy (SRS) scan or gallium-68 Dotatate scans. […] This drug is helpful in treating the symptoms of carcinoid syndrome. Sometimes octreotide can temporarily shrink carcinoid tumors, but it does not cure them. […] The targeted drug, everolimus (Afinitor), has been shown to help treat advanced GI carcinoid tumors. It can be used with or without somatostatin drugs, such as octreotide. […] Interferon-alfa is sometimes helpful in shrinking or slowing the growth of metastatic GI carcinoid tumors and improving symptoms of carcinoid syndrome.

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